Objective To discuss the clinical characteristics of patients withclinical features of patients with neuromyelytis opica and spectrum of neuromyelytis opica(NMOSD) and neuromyelitis optica (NMO).Methods With a retrospective study,From February 2013 to September 2016,a total of 72 NMO patients in Navy General Hospital for diagnosis and treatment were selected as the NMO the NMO group and the other 72 patients of NMOSD patients were selected as the NMOSD group.The results of two groups of patients with general demographic data,ocular symptoms,spinal cord and brain MRI,influence NMO-IgG were recorded.Results There were no significant differences in gender and age compared between the two groups (P ＞ 0.05).The clinical characteristics,frequency and duration in the NMOSD group compared to the NMO group were significantly different (P ＜ 0.05).In the NMOSD group,there were 8 patients who were decreased vision,4 patients were visual field defect,3 patients were discoloration,5 patients were diplopia.While in the NMO group,32 patients were decreased visual acuity,14 patients were visual field defect,12 patients were color vision,21 patients were diplopia.There were statistically significant difference between the two groups (P ＜ 0.05).In the NMOSD group,there were 8 patients were MRI of the spinal cord were normal,64 patients were abnormal and 32 patients of brain lesions in the head MRI.In the NMO group,MRI of the spinal cord were all abnormal,and there were 28 patients were brain lesions in the head MRI.The serum positive rate of NMO group was 41.7％,and the serum positive rate of NMOSD group was 59.7％.The sensitivity of AQP4-Ab antibody to NMO was 44.4％,the specificity was 75％,the sensitivity of AQP4-Ab was 61.1％,and the specificity of NMOSD was 75％.Conclusion The NMOSD is more with female patients,the first symptom is more with the spinal cord that the gray matter involvement in the spinal cord,and the performance of complex,NMO-IgG antibody can be used as a support for NMOSD diagnosis.

Objective To explore the clinical characteristics of infratentorial primary angiitis in central nervous system (PACNS).Methods A total of 5 cases diagnosed as infratentorial PACNS in the neurology department of Navy General Hospital of PLA in 2015 were enrolled in the study.The clinical,imaging and pathological data were collected and analyzed.Results All the 5 cases were male with the median onset age of thirty-four.Five cases presented with dizziness,two with headache,three with walking unstable,two with facial numbness and one with dysarthria.Rising pressure of cerebrospinal fluid (CSF) (190-245 cmH2O,1 cmH2O =0.098 kPa) was found in 4 cases by the lumbar puncture,mildly increased number of leukocyte in 2 cases [(12-28) × 106/L],increased CSF protein in 3 cases(540-979 mg/L) and increased IgG index in 3 cases (0.84-1.45).Pons lesions were revealed by magnetic resonance imaging(MRI) in 4 cases,brachium pontis lesions in 2 cases,cerebellum lesions in 2 cases,one with midbrain lesion in 1 case,unilateral lesions in 4 cases and bilateral lesion in 1 case.Different degree of edema and mass effect were shown in all lesions by MRI.Patch like enhancement was found by contrast MRI in 5 cases and meningeal enhancement in 2 cases.Elevation of choline (Cho) peak was found by magnetic resonance spectroscopy (MRS) in 4 cases,reduction of N-acetyl aspartate (NAA) peak in 3 cases,appearance of lactate peak in 1 case and lipid peak in another case.Arterial spin labeling (ASL) was performed in 4 cases and no hyperperfusion was found.Susceptibility weighted imaging(SWI) was performed in 3 cases and microhemorrhage in the lesions was found in 2 cases and normal in 1 case.Magnetic resonance arteriography(MRA) was performed in 1 case and no stenosis was found.Digital subtraction arteriography(DSA) was performed in 1 case and multiple stenosis of the intracranial arteries was showed.Two cases had taken the stereotactic brain biopsy and the histopathologic diagnosis was angiitis.Five cases were treated with methylprednisolone and cyclophosphamide was added on in 1 case.Good prognosis was found in all cases.Conclusions Infratentorial PACNS mostly attacks middle-aged males.The lesions tend to locate in unilateral pons,brachium pontis,cerebellum and midbrain.Hemorrhage or microhemorrhage in lesions is often found by SWI and no hyperperfusion is shown by ASL,which would be useful to distinguish PACNS from malignant tumors.Given the limitations of brain biopsy in clinical practice,clinical and imaging features would be helpful to diagnose PACNS.

Objective To discover the significance of neurofilament light (NFL) chain of cerebrospinal fluid (CSF), an axonal injury biomarker, in diagnosis and prognosis prediction of neuromyelitis optica spectrum disorders(NMOSD).Methods Sixty-one NMOSD patients and 24 other patients such as neurosis, migraine and so on, with lumbar puncture were enrolled as NMOSD group and normal control (NC) group from in and out patients of Department of Neurology of Navy General Hospital from January 2014 to August 2016. The clinical and neuroimaging features of NMOSD group and CSF samples of both groups were collected,and the NFL levels of CSF were measured by enzyme linked immunosorbent assay. The CSF NFL levels in different subtypes of NMOSD patients were compared, and the influence factors of the NFL levels in CSF were calculated by multiple linear regression analysis. Results The NFL levels of CSF in NMOSD group (2 729.00(14 862.00) pg/ml) were significantly higher than that in NC group ((299.50(308.00) pg/ml, t=8.588, P=0.000;t test of NFL levels was performed after logarithmicly transforming based on 10). There were no statistically significant differences of CSF NFL levels among optic neuritis, longitudinally extensive transverse myelitis and neuromyelitis optica. In NMOSD group, age (b=0.017, P<0.01), Expanded Disability Status Scale score (b=0.078, P<0.05) and enhancement in gadolinium-magnetic resonance imaging (b=0.478, P<0.01) were correlated with the NFL levels of CSF, while gender, courses of diseases and aquaporin 4 antibody in serum were not related to the NFL levels. Conclusion The NFL levels of CSF are conducive to assess the severity and probable progress of NMOSD.

Objective: To study the use of different techniques of pancreaticojejunostomy in laparoscopic pancreaticoduodenectomy (LPD) for patients with slim pancreatic ducts. Methods: The clinical data of 45 patients with slim pancreatic ducts (<3 mm) who underwent LPD in People's Hospital of Zhengzhou University from May 2017 to March 2019 were studied. These patients were divided into 2 groups: Chen's suturing technique (group A, n=31), and the imbedding pancreaticojejunostomy (group B, n=14). The postoperative pancreatic fistula (PF) rates, complication rate, pancreaticojejunostomy time, and length of postoperative hospital stay were compared between the 2 groups. Results: There was no significant difference in the general data between the two groups (P>0.05). The incidence of postoperative pancreatic fistula in group A (4 cases) was significantly lower than that in group B (6 cases) (12.9% vs. 42.9%, P<0.05). The operation time of pancreaticojejunostomy in group A was significantly shorter than that in group B [(26.5±0.8) min vs. (28.0±2.4) min, P<0.05]. There was no significant difference in the length of postoperative hospital stay between the 2 groups (P>0.05). Conclusion Chen's suturing technique was a safe and effective pancreaticojejunostomy technique in LPD carried out in patients with slim pancreatic duct patients.

Objective:To compare the short-term outcomes of patients undergoing laparoscopic pancreaticoduodenectomy (LPD) and those with open pancreaticoduodenectomy (open pancreatiloduodenectomy, OPD).Methods:The clinical data of 85 patients with LPD and 103 patients with OPD at our hospital from Jun 2017 to Jun 2019 were analyzed retrospectively.Results:There was no significant difference in clinical data and between the two groups ( P>0.05). Compared with OPD group, the operation time in LPD group was longer [(407.6±117.4) min vs. (220.8±23.9) min]( P<0.05), but the intraoperative blood loss was less [(285.1±21.9) ml vs. (550.5±65.5) ml]( P<0.05). There was more lymph node dissection (13.5±1.4 vs. 8.8±0.6) ( P<0.05), earlier feeding per month ( P<0.05), shorter time for the use of analgesia and hospital stay ( P<0.05). There was no significant difference in the incidence of postoperative complications such as pancreatic fistula, biliary fistula, gastroparesis and intra abdominal bleeding ( P>0.05). Conclusions:LPD is as safe and reliable as OPD, LPD has the advantages of more precise display during operation, while less traumatic and quicker recovery after operation.

OBJECTIVETo analyze the clinical manifestation of interferon gamma receptor 1 deficiency (IFN-γR1 deficiency) and to improve the recognition of this disease in children, decrease diagnostic errors and missed diagnosis.

METHODThe information of one case with IFN-γR1 deficiency (past history of illness, clinical manifestation, laboratory examination and treatment) were analyzed.

RESULTThe patient was a 19-month-old girl with IFN-γR1 deficiency, 1-2 weeks after she was vaccinated with BCG at the age of 18 months, she manifested with lymph nodes at the same site as vaccination site, and repeated rash. Examination found a mass in the right armpit, the size was 3 cm × 3 cm, protruded on the skin, tenacious in nature, poorly mobile. B-mode ultrasound showed right armpit chest heterogeneous hypoechoic mass; abdominal B-mode ultrasound showed pancreatic lymph nodes around the abdominal aorta and mild swelling; chest X-ray showed right axillary lymph nodes, increased double markings. Initial diagnosis was (1) bronchitis, (2) BCG vaccination reaction, (3) Sepsis? . After admission, the patient was given rifampicin + isoniazid + latamoxef + amoxicillin and clavulanate potassium, and then changed to meropenem and Fusidic acid, but treatment showed no improvement. After adding the treatment with anti-inflammatory treatment, i.e., gamma globulin and methylprednisolone, the fever subsided. Conventional treatment with rifampicin + isoniazid 3 months after discharge from hospital were effective, and the axillary lymph nodes were not palpable. Six months after BCG vaccination bone tuberculosis occurred. CT of left hip and left knee showed bilateral hip joint effusion, left distal femur and left proximal tibia bone destruction. Gene detection showed the presence of homozygous IFNγ-R1 gene mutation of c.114_135del(p.E38fsX54). Her parents are consanguinity, both were carriers. In the literature, 99 cases with IFN-γR1 deficiency were reported, 95% of the cases had disseminated tuberculosis, and in 60 cases the dissemination occurred after BCG vaccination.

CONCLUSIONIFN-γR1 is an extremely rare disease in children. If disseminated tuberculosis infection occured, especially after BCG vaccination, or if there were focal/multifocal bone tuberculosis, immune function with conventional detection is considered normal, then IFN-γR1 deficiency should be considered, and early genetic testing for confirming the diagnosis and selecting the appropriate treatment are needed.

Antitubercular Agents ; therapeutic use ; BCG Vaccine ; adverse effects ; Female ; Humans ; Infant ; Lymph Nodes ; diagnostic imaging ; pathology ; Mutation ; genetics ; Mycobacterium Infections ; diagnosis ; drug therapy ; microbiology ; Receptors, Interferon ; deficiency ; genetics ; Tomography, X-Ray Computed ; Tuberculosis, Osteoarticular ; diagnosis ; drug therapy ; microbiology ; Vaccination ; adverse effects

Novel Coronavirus Pneumonia (NCP) is a class B infectious disease, which is prevented and controlled according to class A infectious diseases. Recently, children′s NCP cases have gradually increased, and children′s fever outpatient department has become the first strategic pass to stop the epidemic. Strengthening the management of the fever diagnosis process is very important for early detection of suspected children, early isolation, early treatment and prevention of cross-infection. This article proposes prevention and control strategies for fever diagnosis, optimizes processes, prevents cross-infection, health protection and disinfection of medical staff, based on the relevant diagnosis, treatment, prevention and control programs of the National Health and Health Commission and on the diagnosis and treatment experience of experts in various provinces and cities. The present guidance summarizes current strategies on pre-diagnosis; triage, diagnosis, treatment, and prevention of 2019-nCoV infection in common fever, suspected and confirmed children, which provide practical suggestions on strengthening the management processes of children′s fever in outpatient department during the novel coronavirus pneumonia epidemic period.